Respiratory insufficiency in patients with progressive muscular dystrophy, in particular dystrophinopathies, develops on the background of many factors. The cause is usually muscular weakness, Severe kyphoscoliosis, the formation of pulmonary atelectases, the incidence of bronchial stenoses, enhanced rezistence of the respiratory system and cardiac failure. The authors present tase-record of three patients with a Severe grade of respiratory insufficiency. The patients have tracheostomies and are in domiciliary ventilation care. The optimal respiratory support is the BiPAP (bilevel positive airway pressure) regimen. The causes of this serious condition are analyzed and attention is drawn to the incidence of bronchial stenoses.

        Key words: progressive muscular dystrophy, respiratory insuficiency, bronchial stenoses